Long-Term Follow-Up at Liver Transplantation

In the majority of cases the children can resume a life close to normal when they leave the hospital and can atted school within 2-3 months of surgery. Clinical and biochemical monitorings are carried out in outpatient clinics; a salt-restricted diet is recommended, at least initially, because of the hipertensive effect of cyclosporine. Since the risk of rejection persists, immunosuppression must be pursued indefinitely, with the goal of reaching the lowest possible doses compatible with normal liver function tests in order to lower the risk of kidney damage due to cyclosporin. Prednisone is given on an alternate day basis, allowing normal growth and a significant increase in height velocity in most children who displayed growth retardation prior to tranplantation. Complications at this stage include late biliary stenosis, opportunistic infections, anemia and gastrointestinal bleeding due to portal vein stenosis, and Epstein Barr Virus (EBV) related lymphoproliferative syndrom. The latter seems in part related to the cumulative degree of immunosuppression, with early diagnosis, lowering or interruption of immunosupression, resection of the proliferative zones when limited and solitary, and careful supervision, regression may occur, but retransplantation may later be necessary because of chronic rejection.

Medium-range follow-up studies indicate that 80% of surviving children have normal serum bilirubin levels and serum transaminase activity below twice normal. Eighty percent of surviving children attend a school level normal for their ages or only 1 year below normal. A significant prognostic factor is the condition of the child before transplantation, both in term of nutrition and liver cell function. For instance among 284 children treated in our group between 1986 and 1993, survival was 57% in the children whose pretransplant prothrombin time was below 50%, and 93% in the children whose prothrombin time was equal to or above 50%. In particular all children transplanted for an inborn error of metabolism without severe liver damage are currently alive.